Table of Contents
1
UI - 11794989
AU - Gallet B; Quintard H; Fruchaud J; Hiltgen M
TI -
[Dynamic left ventricular obstruction associated with a pheochromocytoma
reversible after tumor ablation]
SO - Arch Mal Coeur Vaiss 2001 Nov;94(11):1195-8
AD - Service de cardiologie, centre hospitalier Victor-Dupouy, 69, rue du
Lieutenant-Colonel-Prudhon, 95107 Argenteuil.
The association of dynamic left ventricular outflow tract obstruction
and pheochromocytoma is rare with only about fifteen previously reported
cases in the literature. The association may be the result of chronic
increased catecholamine secretion which induces intra-ventricular
obstruction in a hypertrophied ventricle due to hypertension. The
authors report a new case in which the intraventricular gradient on
Doppler echocardiography was particularly high at 120 mmHg. The gradient
totally regressed after removal of the pheochromocytoma. The finding of
a systolic murmur in a patient with pheochromocytoma should lead to the
prescription of Doppler echocardiography and diagnosis of left
ventricular tract obstruction must lead to the association of
betablocker and alphablocker therapy before surgery. The removal of the
pheochromocytoma is usually followed by the complete regression of left
intraventricular obstruction, as in this case.
2
UI - 11803636
AU - Chapuis Y; Dousset B; Bonnichon P; Massault PP; Hoeffel C; Louvel A
TI -
[Which adrenal incidentalomas are to be operated?]
SO - Ann Chir 2001 Dec;126(10):985-91
AD - Service de chirurgie generale et digestive, hopital Cochin, 27, rue du
Faubourg-Saint-Jacques, 75674 Paris, France. yveschapuis@clubinternet.fr
AIM OF THE STUDY: The goal of this retrospective study was to recognize
the incidence of adrenal tumors called incidentalomas, that are in fact
symptomatic or hormone secreting tumors, to assess if the diagnostic
criteria proposed in the literature are reliable for adrenal tumors
found incidentally. PATIENTS AND METHOD: The records concerned 147
mass measuring 3 cm in diameter and larger on CT scan. Clinical and
laboratory findings as well as hormone levels and imaging examinations
(CT Scan, IRM, scintigraphy) were correlated with operative findings and
results of histologic examinations. One hundred and three patients were
operated by a traditional approach and forty four by videoendoscopic
techniques. RESULTS: In 41 patients (28%), the tumors had to be
classified as false incidentalomas (group 1) postoperatively. In 106
patients (72%), the nature of tumor was non definite. In this group 2,
there were ten malignant tumors, two pheochromocytomas and 94 benign
lesions. Age of patient, size of tumor, increase in size over time and
radiological appearance represent criteria that are not reliable to
separate malignant from benign tumors. CONCLUSIONS: Tumors of
indeterminated and non specific nature only should be classified as
incidentalomas. Imaging techniques as well as other criteria of
malignancy cannot be relied upon to separate benign from malignant
tumors. The risk even small of cancer or pheochromocytoma leads us to
recommend excision of tumors > or = 3 cm in radiological diameter. The
videoendoscopic approach is a good alternative to open operation in
centers with this special expertise.
3
UI - 11809186
AU - Ma RC; Chan WB; Chow CC; Cockram CS
TI -
A woman with vomiting and hyperamylasaemia.
SO - Lancet 2002 Jan 5;359(9300):42
AD - Department of Medicine and Therapeutics, Prince of Wales Hospital,
Shatin, New Territories, Hong Kong. rcwma@netvigator.com
4
UI - 11795252
AU - Smith SL; Slappy AL; Fox TP; Scolapio JS
TI -
Pheochromocytoma producing vasoactive intestinal peptide.
SO - Mayo Clin Proc 2002 Jan;77(1):97-100
AD - Section of General Surgery, Mayo Clinic, Jacksonville, Fla 32224, USA.
smith.stephen@mayo.edu
The syndrome of watery diarrhea associated with hypokalemia and
achlorhydria was originally described in 1958. Subsequently, this
syndrome was shown to be caused by a neuroendocrine tumor secreting
vasoactive intestinal peptide (VIP), and such tumors are almost always
pancreatic in origin. We describe the case of a 78-year-old woman with
gradual onset of hypokalemia, watery diarrhea, and weight loss. After a
left adrenal mass was discovered, the patient chose medical therapy over
surgical intervention. Initially her condition responded, then gradually
became refractory to medical therapy. She had elevated levels of VIP,
pancreatic polypeptide, dopamine, and vanillylmandelic acid.
Subsequently, the patient underwent surgical excision of the mass that
was found to be a VIP-producing pheochromocytoma. After surgery her
diarrhea subsided, and her electrolytes and affected neuroendocrine
hormone levels normalized.
5
UI - 11821960
AU - Koch CA; Huang SC; Zhuang Z; Stolle C; Azumi N; Chrousos GP; Vortmeyer
TI -
AO; Pacak K
Somatic VHL gene deletion and point mutation in MEN 2A-associated
pheochromocytoma.
SO - Oncogene 2002 Jan 17;21(3):479-82
AD - Pediatric and Reproductive Endocrinology Branch, National Institute of
Child Health and Human Disease (NICHD), National Institutes of Health
(NIH), Bethesda, Maryland, MD 20892, USA. kochc@exchange.nih.gov
Multiple endocrine neoplasia type 2 (MEN 2) is an inherited cancer
syndrome that includes pheochromocytoma. Germline mutations in RET are
responsible for MEN 2 but the precise pathogenetic mechanisms of
tumorigenesis are unknown. We have recently identified possible
mechanisms of tumor formation in patients with MEN 2A-related
pheochromocytoma. Two of nine tumors investigated, however, did not
reveal either of these mechanisms. In the present study, we therefore
searched for other possible mechanisms underlying the pathogenesis of
MEN 2A-related pheochromocytoma. Hereditary pheochromocytoma also occurs
in patients with von Hippel-Lindau (VHL) disease, a syndrome consisting
of tumors caused by inactivation of the VHL tumor suppressor gene. A
subset of sporadic pheochromocytomas have somatic mutations in RET or
VHL, suggesting that both genes contribute to pheochromocytoma
pathogenesis in a subset of tumors. It is unknown, however, whether VHL
gene alterations would be associated with tumorigenesis in hereditary,
MEN 2-related pheochromocytoma. We therefore investigated four
pheochromocytomas from patients with MEN 2A and RET germline mutations
for the presence of allelic deletion and/or somatic mutation of the VHL
gene. LOH analysis using the polymorphic markers D3S1038 and D3S1110
that map to the VHL gene locus 3p25/26, revealed evidence for somatic
VHL gene deletion in all four MEN 2A-related pheochromocytomas. Mutation
analysis of the VHL gene showed frameshift mutations in two tumors and a
splice acceptor mutation in one tumor. The remaining tumor did show LOH
but not mutation of the VHL gene. These results suggest that somatic
genetic alterations of the VHL gene may play a role in the tumorigenesis
of some MEN 2A-related pheochromocytomas.
6
UI - 11834382
AU - Hsu TH; Gill IS
TI -
Bilateral laparoscopic adrenalectomy: retroperitoneal and
transperitoneal approaches.
SO - Urology 2002 Feb;59(2):184-9
AD - Section of Laparoscopic and Minimally Invasive Surgery, Urological
Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
OBJECTIVES: To report our experience with bilateral laparoscopic
adrenalectomy using either the retroperitoneal or the transperitoneal
patients with bilateral adrenal disease (Cushing syndrome in 4,
pheochromocytoma in 1, and solitary adrenal metastases in 1) underwent
bilateral laparoscopic adrenalectomy by way of the retroperitoneal or
transperitoneal approach. Bilateral adrenalectomy was performed either
synchronously (4 patients) or in a staged manner (2 patients). RESULTS:
All laparoscopic procedures were performed successfully. For the
bilateral synchronous cases (n = 4) and metachronous cases (n = 4), the
median surgical time was 278 and 195 minutes, blood loss was 175 and 125
mL, resumption of oral intake and ambulation required less than 1 day
for both groups, and postoperative narcotic requirement comprised 14 and
10 mg morphine sulfate equivalent, respectively. For the synchronous and
metachronous cases, the hospital stay was 1.5 and less than 1 day and
convalescence was 3 and 2.5 weeks, respectively. An intraoperative
surgical complication (adrenal vein bleeding) occurred in 1 case, but
did not require open conversion. No postoperative complications or
rehospitalization occurred. The retroperitoneal approach was used in 3
of the 4 bilateral synchronous and 3 of the 4 bilateral metachronous
cases. In both synchronous and metachronous cases, the actual surgical
time, blood loss, time to oral intake and ambulation, postoperative
analgesia use, hospital stay, and convalescence of the transperitoneal
approach were well within the range of those of the retroperitoneal
approach. CONCLUSIONS: Retroperitoneal and transperitoneal bilateral
laparoscopic adrenalectomy is feasible, safe, and results in minimal
postoperative morbidity.
7
UI - 11786741
AU - Boersma HH; Wensing JW; Kho TL; De Brauw LM; Liem IH; Van Kroonenburgh
TI -
MJ
Compensatory uptake of I-123 MIBG in the contralateral adrenal gland
after removal of a pheochromocytoma.
SO - Clin Nucl Med 2002 Feb;27(2):113-6
AD - Department of Nuclear Medicine, University Hospital Maastricht,
Maastricht, The Netherlands.
The identification of recurrent or residual tumor tissue is sometimes
complicated. The authors describe a 53-year-old woman in whom I-123
metaiodobenzylguanidine (MIBG) scintigraphy revealed a pheochromocytoma
in the right adrenal gland. After the tumor was removed, the patient's
catecholamine levels normalized. At the 3-month follow-up examination,
I-123 MIBG scintigraphy did not reveal uptake in the right adrenal
region but rather showed uptake in the left adrenal region. The
patient's blood pressure remained in the normal range. A third
scintigram, obtained 1 year after tumor resection, no longer detected
I-123 MIBG accumulation in the left adrenal gland. These findings
suggest that compensatory hyperplasia of the left adrenal gland led to
enhanced uptake of I-123 MIBG. They also highlight the need for careful
follow-up of such patients to distinguish between physiologic and
pathologic processes.
8
UI - 11677427
AU - Gentile S; Rainero I; Savi L; Rivoiro C; Pinessi L
TI -
Brain metastasis from pheochromocytoma in a patient with multiple
endocrine neoplasia type 2A.
SO - Panminerva Med 2001 Dec;43(4):305-6
AD - Department of Neurology III, Headache Center Department of Neuroscience,
University of Turin, Turin, Italy. pinessi@medfarm.unito.it
Neurological involvement in multiple endocrine neoplasia (MEN) syndrome
is uncommon. Notalgia paresthetica (pruritus localized in an area
between D2 and D6 dermatomes) is the neurological symptom more
frequently described in patients with MEN 2A. The authors report the
unusual case of a MEN 2A patient with a brain metastasis from a
pheochromocytoma.
9
UI - 11754954
AU - Takahashi K
TI -
Adrenomedullin: from a pheochromocytoma to the eyes.
SO - Peptides 2001 Nov;22(11):1691
10
UI - 11404820
AU - Astuti D; Latif F; Dallol A; Dahia PL; Douglas F; George E; Skoldberg F;
TI -
Husebye ES; Eng C; Maher ER
Gene mutations in the succinate dehydrogenase subunit SDHB cause
susceptibility to familial pheochromocytoma and to familial
paraganglioma.
SO - Am J Hum Genet 2001 Jul;69(1):49-54
AD - Section of Medical and Molecular Genetics, Department of Paediatrics and
Child Health, University of Birmingham, Birmingham B15 2TT, United
Kingdom.
The pheochromocytomas are an important cause of secondary hypertension.
Although pheochromocytoma susceptibility may be associated with germline
mutations in the tumor-suppressor genes VHL and NF1 and in the
proto-oncogene RET, the genetic basis for most cases of nonsyndromic
familial pheochromocytoma is unknown. Recently, pheochromocytoma
susceptibility has been associated with germline SDHD mutations.
Germline SDHD mutations were originally described in hereditary
paraganglioma, a dominantly inherited disorder characterized by vascular
tumors in the head and the neck, most frequently at the carotid
bifurcation. The gene products of two components of succinate
dehydrogenase, SDHC and SDHD, anchor the gene products of two other
components, SDHA and SDHB, which form the catalytic core, to the
inner-mitochondrial membrane. Although mutations in SDHC and in SDHD may
cause hereditary paraganglioma, germline SDHA mutations are associated
with juvenile encephalopathy, and the phenotypic consequences of SDHB
mutations have not been defined. To investigate the genetic causes of
pheochromocytoma, we analyzed SDHB and SDHC, in familial and in sporadic
cases. Inactivating SDHB mutations were detected in two of the five
kindreds with familial pheochromocytoma, two of the three kindreds with
pheochromocytoma and paraganglioma susceptibility, and 1 of the 24 cases
of sporadic pheochromocytoma. These findings extend the link between
mitochondrial dysfunction and tumorigenesis and suggest that germline
SDHB mutations are an important cause of pheochromocytoma
susceptibility.
11
UI - 11850829
AU - Lui WO; Chen J; Glasker S; Bender BU; Madura C; Khoo SK; Kort E; Larsson
TI -
C; Neumann HP; Teh BT
Selective loss of chromosome 11 in pheochromocytomas associated with the
VHL syndrome.
SO - Oncogene 2002 Feb 7;21(7):1117-22
AD - Department of Molecular Medicine, Karolinska Hospital, Stockholm,
Sweden.
By using comparative genomic hybridization (CGH), we characterized the
genetic profiles of 36 VHL-related pheochromocytomas. We then compared
the results with those of sporadic and MEN 2-related pheochromocytomas.
In 36 VHL-related tumors, loss of chromosome 3 and chromosome 11 were
found in 34 tumors (94%) and 31 tumors (86%), respectively. There was
significant concordance of deletions in chromosomes 3 and 11
(Kappa=0.64, P=0.0095), suggesting that they are involved in two
different but necessary and complementary genetic pathways. The loss of
chromosome 11 appeared to be specific for VHL-related pheochromocytoma
as it was not present in any of the 10 VHL-related CNS hemangioblastomas
studied and was significantly less common when compared with (a)
sporadic pheochromocytomas from previously published results (13%;
P=<0.0001), and (b) MEN 2-related pheochromocytomas from this and
previously published studies (30%; P=0.0012). In summary, this is the
first report of a novel consistent genetic alteration that is selected
and specific for VHL-related pheochromocytoma, besides the two hits of
the VHL gene.
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